Hypoplastic Left Heart Syndrome: About a Postnatal Death.

Area of Science:

• Cardiology
• Pediatric Cardiology
• Forensic Pathology

Background:

• Hypoplastic left heart syndrome (HLHS) is a severe congenital heart defect associated with high neonatal mortality.
• Missed prenatal diagnosis and delayed suspicion contribute to poor therapeutic outcomes in HLHS.
• Congenital heart disease management is challenged by diagnostic delays.

Purpose of the Study:

• To present a case of a neonate with undiagnosed Hypoplastic Left Heart Syndrome (HLHS).
• To highlight the medico-legal implications of missed congenital heart disease diagnoses.
• To emphasize the importance of prenatal diagnosis in HLHS management.

Main Methods:

• A forensic autopsy was conducted on a female neonate who died 26 hours after birth due to respiratory failure.
• Macroscopic examination of the heart was performed to identify cardiac abnormalities.
• Review of intrauterine medical history for documented cardiac or genetic conditions.

Main Results:

• Autopsy revealed severe hypoplasia of the left heart chambers, with a rudimentary left ventricle.
• The right ventricular cavity appeared enlarged, simulating a single ventricle.
• Macroscopic findings were consistent with HLHS, despite no prenatal diagnosis.

Conclusions:

• Hypoplastic Left Heart Syndrome (HLHS) is a life-limiting condition with high mortality due to cardiorespiratory insufficiency shortly after birth.
• Prompt prenatal diagnosis of HLHS is essential for effective management and surgical planning.
• This case underscores the critical need for improved prenatal screening for congenital heart defects like HLHS.